title: Oral steroids for long-term use in cystic fibrosis.
abstract: BACKGROUND
  In cystic fibrosis (CF) airway obstruction and recurrent respiratory infection lead to inflammation, long-term lung damage, respiratory failure and death. Anti-inflammatory agents, e.g. oral corticosteroids are used since inflammation occurs early in disease.
  
  
  OBJECTIVE
  To assess the effectiveness of oral corticosteroids in respiratory complications in CF, particularly lung function and adverse events. We examined long-term use (over 30 days) only.
  
  
  METHODS
  We searched the Cochrane CF and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Most recent search: 15 May 2013.
  
  
  METHODS
  Randomised trials comparing oral corticosteroids given for more than 30 days with placebo or no additional therapy in people with CF.
  
  
  METHODS
  Two authors independently assessed study eligibility and quality.
  
  
  RESULTS
  Of eleven studies identified, three (354 participants) were included: two with four-year follow up and one with 12-weeks follow up. Data were lacking on predefined outcomes; common outcomes were examined at different time-points and presented differently. Meta-analyses were not possible.In one study, oral corticosteroids at prednisolone-equivalent dose of 1 mg/kg alternate days slowed progression of lung disease; at two and four years, % predicted FEV1 in the 1 mg/kg group changed significantly more than in the placebo group (P < 0.02). During the first two years, the 2 mg/kg group was not significantly different from the placebo group. Linear growth retardation was observed from six months in the 2 mg/kg alternate days prednisolone group and from 24 months in the 1 mg/kg alternate days prednisolone group.Adverse events terminated one four-year study early. Year 10 follow up showed catch-up growth started two years after treatment ceased. Alternate-day treatment with oral corticosteroids may have impaired growth until adulthood in boys.
  
  
  CONCLUSIONS
  Oral corticosteroids at prednisolone-equivalent dose of 1 to 2 mg/kg alternate days appear to slow progression of lung disease in CF; benefit should be weighed against occurrence of adverse events. Risk-benefit analysis of low-dose alternate days corticosteroids is important and the short-term use of oral corticosteroids should be better evaluated.
authors.affiliation: c/o Cochrane Cystic Fibrosis & Genetic Disorders Review Group, Institute of Child Health, University of Liverpool, Liverpool, UK. nikkij@liv.ac.uk.
authors.forename: Katharine
authors.initials: K
authors.lastname: Cheng
authors.forename: Deborah
authors.initials: D
authors.lastname: Ashby
authors.forename: Rosalind L
authors.initials: RL
authors.lastname: Smyth
mesh_terms: D000284:Administration, Oral; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage* / Q000009:adverse effects; D002648:Child; D003550:Cystic Fibrosis / Q000150:complications*; D057239:Early Termination of Clinical Trials; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage* / Q000009:adverse effects; D006128:Growth / Q000187:drug effects; D006801:Humans; D008297:Male; D011239:Prednisolone / Q000008:administration & dosage / Q000009:adverse effects; D016032:Randomized Controlled Trials as Topic; D012140:Respiratory Tract Diseases / Q000188:drug therapy* / Q000209:etiology; D012737:Sex Factors
chemical_list: D000893:Anti-Inflammatory Agents; D005938:Glucocorticoids; D011239:Prednisolone